November 17th 2010 Questions you should be able to answer after this session What is the most common movement disorder?
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Robert Altman November 17th 2010
Questions you should be able to answer after this session What is the most common movement disorder? Name 2 drugs that can enhance a physiologic tremor and 2 that can turn someone akinetic and rigid? What is a first line therapy for early tremor in PD for a 45 yr old? What are the 2 P’s for essential tremor treatment? Which tremor is so stirkingly unilateral and disabling that the patient’s limb is deemed non-functional? Tensor or levator in essential palatal tremor?References AAN Movement Disorders Syllabus, 2010 Toronto AAN Continuum 2007, 2010 Movement Disorders Videos courtesy of YouTube and AAN Continuum CD As usual; good review articles Tremor : Clinical Features, Pathophysiology, and Treatment: Neurol Clin 27 (2009) 679–695 Grimes DA. Tremor--easily seen but difficult to describe and treat. Can J Neurol Sci. 2003 Mar;30 Suppl 1:S59-63. Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol. 2009 Aug;22(4):430-6. Contemplate... Definition of tremor Rational and logical categorization Examples of each Therapies for each Focus in essential tremor Definition Genetics Clinical Pharmacological and non-pharma treatments Surgical therapies (refractory cases) What / where to lesion or stimulate? Psychogenic Movement Disorders* Definition: Tremor Movement of a body part Involuntary (even PMD) Rhythmical Regularly recurrent Oscillatory Around central plane Categorization Previously described in terms of activation condition (rest vs. movement), brain region affected (rubral), presence or absence of medical condition(hypothyroid), existence of neurological conditions (dystonia), topography (limb, head, voice), frequency..... Complicated and contradictory Generally not necessary to diagnose and properly treat Phenomenology and a syndromic classification of tremor consensus statement Movement Disorder Society (MDS) [1998]Treat the correct cause
TREMOR
3 golden rules in tremor assessment
Action Most Common :Essential tremor Enhanced physiological tremor Drug-Induced action tremor Dystonic Less Common: Orthostatic Cerebellar Psychogenic Wilsonian FXTAS Peripheral neuropathy-related Midbrain or rubral
On history Tremor causing Rx? Caffeine, nicotene? Diarrhea, weight loss, heat intolerenace? Sudden onset? Temporal course? Static or progressive? What body regions? On exam Postural / sustension: What joints? Thumb posturing? Other abnormal postures? Distractibility, entrainment, suggestibility? Kinetic part: Pouring, drinking, using a spoon, FNF, Archimedes spiral test Intentional component (worsen as draw near target) Re-emergent? Dystonic postures accompanying? (thumb) Essential Tremor Most common adult-onset movement disorder 5% general population Genetics: AD, variable penetrance, no gene found (polygenic) Central generator: thought to represent cerebellar-thalamo-cortical outflow pathology Kinetic and postural, mainly arms; 4-12Hz Progressive Armshead (“yes-yes” vs. “no-no”) Voice / vocal cord, chin, tongue Unilateral bilateral Rare in LE’s No parkinsonian or dystonic features. Disability Interferes with ADL’s Feeding Spoon, drinking from a cup Writing Typing Personal hygiene Interferes w/ occupational motor tasks Diagnostic Criteria Core Bilateral action tremor of the hand and forearms Absence of other neurologic signs Caveat: cogwheeling* May have isolated head tremor with no signs of dystonia Secondary / Supportive Long duration (3 yrs) + family Hx Beneficial response to ETOH 50-90% of cases, but careful for rebound phenomenon Non-motor ET symptoms Non-motor (tremor) symptoms being recognized Implications for screening, treatment plans Archimedes Spiral Test
Treatment of ET General principals Treat only if bothersome The longer the tremor has been there the more difficult Tx will be Limb tremor responds much better than head/neck to oral RxNon-pharmacologic Biofeedback Weighted objects (e.g., utensils) Only dampens it temporarily, not viable long term treatment option Pharmacologic 2 P’s ; alone or in combo. Is there concurrent HTN? Primidone, propranolol (Inderal) Others: benzodiazepenes, gapapentin anecdotal use but not class A evidence (see table in appendix) Botox Voice, head DBS (not lesional) ViM of VL of thalamus in refractory cases 2 P’s
Enhanced Physiologic Tremor (EPT) Appears to be peripherally generated (not central like ET) Based on inertial loading electrophysiological analysis Faster postural and kinetic tremor than ET (7-12Hz); very low amplitude. Very easily visible 15-35 yo Anxious phenotype Mainly voice and limb No head May have some cogwheeling, no frank rigidity Endogenous & exogenous (see next slide) causes Tx Drug Induced Action Tremor (EPT)Based on history Temporal onset Sympathomimetics++ (ß -adrenergic bronchodilators) caffeine, nicotene SSRI, Li, valproate, roids! Withdrawal relieves symptoms Limb, never head Treatment Remove causative agent Bb or bzdp can dampen tremor if causative Rx absolutely necessary Dystonic Tremor Tremulous Pulling or pain sensation in region affected (e.g., neck) Limbs (UE>LE), head (neck) or both (limb precedes neck) Voice: strangled speech , voice break Postural or kinetic Not rythmic, nor oscillatory, not around 1 axisExam may reveal tonic muscle activity in tremoulous or conta-tremulous limb Tremulousness is directional Spooning fatiguing , thumb flexion dystonic postures May have scarf hiding hypertrophied muscle (eg neck with toriticollis) Dystonic tremor may be reduced by antagonistic gestures geste antagonistique Commonly misdiagnosed as ET Pharma treatment: anti-dystonic agents (baclofen, artane), bzdp, bb, botox (torticollis, voice) Surgical (refractory): selective denervation, DBS Orthostatic Tremor Rare Avoid situations when have to stand still (at movies) o/e Rapid 13-18hz, low amplitude tremor/rippling in calves only on standing Visible and palpable pseudodystonic Confirmed with EMG Rx: Nothing evidence based Most common =Clonazepam, sinemet Cerebellar Tremor Central cerebellar disorders (e.g.,SCA) Kinetic with terminal worsening = intentional May have postural component, but rest absent In multiple planes Slow, 3-5 hz Presence of overshooting Other cerebellar signs (hypermetric saccades, dysarthria, scanning speech, ataxic gait, head titubation, dyssynergia) Treatment Wilson’s Disease associated Tremor Can be action (rest, combination) Postural (possibly wing-beating) Kinetic (like ET) Young (<40 yo) Exam reveals multifocal and multisystemic disorder (long-tract, cognitive, neuro-psychiatric) Trivia: what are the genetics? Mode of transmission? On what layer of the cornea would you look to find KF rings? Tx Chelation Penicillamine controversial Trientene Tetrathiomolybate Zinc once levels normalize Bb for action tremor FXTAS (fragile X tremor ataxia syndrome) Cerebellar or parkinsonian Cognition, dysexectuive function Male family members (grandchildren) have MR MR brain Tx: bb for action tremor Peripheral neuropathy-related tremor PN by history of same limb with tremor Intertial loading leads to suppression of tremor proving peripheral generator More common with demyelinating PN’s, also seen in HMSN1 (Levy-Roussy) and IgM dysgammaglobulinemic neuropathies Temporal linkage o/e Peripheral neuropathy readily apparent on tremulous limb Sensory impairment, weakness 4/5 MRC, altered DTR Tremor present when muscle strength allows limb to maintain certain postures Vanishes if weakness too severe or if limb power returns to normal Tx: Underlying neuropathy BB for AT What do you think about DBS? Midbrain / Rubral / Holmes Lesion based, central generator Sudden onset focal neurological insultProgressive forms can occur (tumor or expanding vascular lesion)..some say this is typical even for stroke. Strikingly unilat , HB or monomelicNon-rythmic, <4.5 Hz, high amplitude Rest, action (postural and kinetic) Severity: kinetic > postural > rest Severe and disabling, limb entirely handicapped Imaging confirms pontine-midbrain lesion affecting cerebellar outflow tracts and dopaminergic nigrostriatal fibers Rx: AT primidone, bb Rest Levodopa, DA, Anti-chol DBS (refratory) Palatal “myoclonus” – actually tremor Essential vs. symptomatic See Chenjie’s presentation, great comparative table Rest Tremor(s) Most Common :Parkinsonian Drug-induced rest tremor ET (with rest component; rare…15-20%) Less Common: Critical Elements from Hx & Exam History Rx? Change in arm swing, gait, facial expression? Previous CVA, dementia? Exam Arms at rest (whole interview and dynamic exam) ‘pill-rolling’ quality Symmetry Limb or hemi-body Arm extension test Not true postural, rather emergent Parkinsonian Tremor Classically @ rest; 3-5 Hz Often HB If arm Pron-sup rather than flx-ext Check for limb “posturing” flexion/fist formation hand, thumb flexion Re-emergent tremor during arm extension or during tasks (pouring water) causes considerable misdiagnosis with ET Other hallmark-cardinal features Motor : asymmetric rigidity, bradykinesia, postural instability, fatiguingNon-motor : RBD, hyposmia, constipation, mood, sebborhea, ANS dysfunction (orthostasis, ED, etc.), excessive daytime sleepiness, RLS, body pain .......Aversion to caffeine...? PD tremor DA 1st line Levodopa Anticholinergics (rare) Cogentin, artane, amantadine Consider bzdp Refractory DBS Drug Induced Temporal link with Rx Antipsychotics (typical>atypical), Li Can look practically identical Removal of medication should result in complete resolution Tx: Remove or diminish offending agent Levodopa (even if on Da blocking agents) or anticholinergics can be tried Psychogenic Tremor (PMD) History
Psychogenic Movement Disorders (PMD )
PMD Not a diagnosis of exclusionShould be recognized and treated rapidly to avoid stigmatization, ‘crazy’ label CBT, neurologist (a movement disorder induced by internal stress), psychiatrist (somatization) Frequency of PMD in clinical practice
Questions you should be able to answer after this session What is the most common movement disorder? Name 2 drugs that can enhance a physiologic tremor and 2 that can turn someone akinetic and rigid? What is a first line therapy for early tremor in PD for a 45 yr old? What are the 2 P’s for essential tremor treatment? Which tremor is so strikingly unilateral and disabling that the patient’s limb is deemed non-functional? Tensor or levator in essential palatal tremor?Thanks!
Palatal Myoclonus saga... Palatal Myoclonus : involvement of central tegmental tracts Dostları ilə paylaş:
